Our previous work has highlighted the implication of ERAD in the loss of function mutations in some TGFβ receptors such as endoglin, Activin Receptor-Like Kinase-1 (ALK1), and Bone morphogenetic protein receptor (BMPR2), which cause Hereditary Haemorrhagic Telangiectasia (HHT1 and HHT2) and familial pulmonary arterial hypertension, respectively (Ali et al., 2011; Hume et al., 2013; Gariballa et al., 2022). Here, ACVRL1 is linked to heritable pulmonary arterial hypertension.